PRNP and inherited Creutzfeldt-Jakob disease: However, Zou and co-workers recently discovered that the sporadic VPSPr along with familial Creutzfeldt-Jakob disease (fCJD) associated with a PrP mutation Val to Ile at residue 180 (fCJDV180I) generates a PrPSc molecule with a unique electrophoretic gel profile and is characterized by the accumulation in the brain of PrPSc that lacks diglycosylated PrPSc and PrPSc monoglycosylated at residue 181 [4–6].